191 results match your criteria Sickle Cell Anemia Skeletal


Skeletal Muscle Satellite Cells in Sickle Cell Disease Patients and Their Responses to a Moderate-intensity Endurance Exercise Training Program.

J Histochem Cytochem 2022 Jun 31;70(6):415-426. Epub 2022 May 31.

Univ Lyon, UJM-Saint-Etienne, Laboratoire Interuniversitaire de Biologie de la Motricité, Saint-Etienne, France.

We previously demonstrated that 8 weeks of moderate-intensity endurance training is safe and improves muscle function and characteristics of sickle cell disease (SCD) patients. Here, we investigated skeletal muscle satellite cells (SCs) in SCD patients and their responses to a training program. Fifteen patients followed the training program while 18 control patients maintained a normal lifestyle. Read More

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Renin-Angiotensin Blockade Reduces Readmission for Acute Chest Syndrome in Sickle Cell Disease.

Cureus 2022 Mar 28;14(3):e23567. Epub 2022 Mar 28.

Pathology, University of Missouri-Kansas City, Kansas City, USA.

Rationale Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease (SCD). Current treatment is supportive-supplemental oxygen, transfusions, and antibiotics. Prevention of ACS may reduce morbidity and mortality in patients with SCD. Read More

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Hyaluronic acid injections for treatment of pediatric sickle cell avascular necrosis of the humeral head.

Reg Anesth Pain Med 2022 02 12;47(2):136-138. Epub 2021 Oct 12.

Department of Anesthesiology and Critical Care Medicine, Children's Hospital of Los Angeles, Los Angeles, California, USA.

Background: Avascular necrosis (AVN) can impact up to 50% of patients with sickle cell disease (SCD) and can result in significant pain, decline in physical function and decreased quality of life. While hyaluronic acid (HA) has been used in the adult population for shoulder osteoarthritic pain, we present the first published pediatric case of HA injections in the glenohumeral joint, used to improve function and pain control.

Case Presentation: The patient is a 12-year-old woman with SCD, who suffered from chronic pain due to AVN of the humeral and femoral head. Read More

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February 2022

Core Decompression and Bone Marrow Aspirate Concentrate in the Treatment of Femoral Head Avascular Necrosis in Pediatric Sickle Cell Disease: Can We Improve Natural History?

J Pediatr Orthop 2021 Nov-Dec 01;41(10):604-609

Children's Healthcare of Atlanta, Scottish Rite, Sandy Springs, GA.

Introduction: Femoral head avascular necrosis (AVN) affects between 10% and 41% of children with sickle cell disease (SCD), resulting in the development of proximal femoral deformity and residual hip pain in the majority of patients without treatment. There have been multiple adult studies published on the outcomes of core decompression with and without the use of bone marrow aspirate concentrate (BMAC) injection both in idiopathic and SCD-related AVN with promising results, however, few studies have reported on outcomes in skeletally immature patients.

Purpose: This study sought to report on a single surgeon's outcomes for core decompression with BMAC injection in pediatric patients with AVN of the femoral head secondary to underlying SCD. Read More

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October 2021

Cerebral and skeletal muscle tissue oxygenation during exercise challenge in children and young adults with sickle cell anaemia.

Br J Haematol 2022 01 26;196(1):179-182. Epub 2021 Jul 26.

Division of Hematology, Oncology and Stem Cell Transplant, Department of Pediatrics, Ann & Robert H Lurie Children's Hospital of Chicago, Chicago, Illinois, USA.

We used near-infrared spectroscopy to examine tissue oxygenation (StO during exercise in 17 children and young adults with sickle cell anaemia (SCA) and 13 controls. Patients had lower cerebral StO at all exercise stages and demonstrated significantly greater decreases in cerebral StO later during exercise. Quadriceps StO increased similarly in patients and controls during early exercise, but decreases from baseline were greater in patients during later exercise. Read More

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January 2022

Is Skeletal Muscle Dysfunction a Limiting Factor of Exercise Functional Capacity in Patients with Sickle Cell Disease?

J Clin Med 2021 May 22;10(11). Epub 2021 May 22.

Inter-University Laboratory of Human Movement Sciences (LIBM) EA7424, Team "Vascular Biology and Red Blood Cell", University Claude Bernard Lyon 1, 69100 Villeurbanne, France.

Patients with sickle cell disease (SCD) have reduced functional capacity due to anemia and cardio-respiratory abnormalities. Recent studies also suggest the presence of muscle dysfunction. However, the interaction between exercise capacity and muscle function is currently unknown in SCD. Read More

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Impact of a submaximal mono-articular exercise on the skeletal muscle function of patients with sickle cell disease.

Eur J Appl Physiol 2021 Sep 22;121(9):2459-2470. Epub 2021 May 22.

Inter-University Laboratory of Human Movement Sciences (LIBM) EA7424, Team "Vascular Biology and Red Blood Cell", University Claude Bernard Lyon 1, Villeurbanne, France.

Purpose: Sickle cell disease (SCD) patients exhibit a limited exercise tolerance commonly attributed to anaemia, as well as hemorheological and cardio-respiratory abnormalities, but the functional status of skeletal muscle at exercise is unknown. Moreover, the effect of SCD genotype on exercise tolerance and skeletal muscle function has been poorly investigated. The aim of this study was to investigate skeletal muscle function and fatigue during a submaximal exercise in SCD patients. Read More

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September 2021

Occlusal Disorders in Patients with Sickle Cell Disease: Critical Literature Review.

J Clin Pediatr Dent 2021 Apr;45(2):117-122

Objective: To identify the association of occlusal disorders in patients with sickle cell disease (SCD).

Study Design: A literature review was conducted, and articles published between 2010 and 2019 were searched on Bireme and PubMed websites and in MEDLINE and LILACS databases, in English, Portuguese, and Spanish, using the keywords "malocclusion," "sickle cell disease," and "cephalometry," combined by Boolean operators AND and OR. One of the criteria for the selection of articles was the presence of adolescents in the sample. Read More

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Sickle Cell Trait Modulates the Proteome and Phosphoproteome of -Infected Erythrocytes.

Front Cell Infect Microbiol 2021 24;11:637604. Epub 2021 Mar 24.

Université de Paris, MERIT, IRD, Paris, France.

The high prevalence of sickle cell disease in some human populations likely results from the protection afforded against severe malaria and death by heterozygous carriage of HbS. remodels the erythrocyte membrane and skeleton, displaying parasite proteins at the erythrocyte surface that interact with key human proteins in the Ankyrin R and 4.1R complexes. Read More

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Intravenous Acetaminophen vs Intravenous Diclofenac Sodium in Management of Skeletal Vaso-occlusive Crisis Among Children with Homozygous Sickle Cell Disease: A Randomized Controlled Trial.

Indian Pediatr 2021 03;58(3):229-232

Department of Community Medicine, VSSIMSAR, Burla, Sambalpur, Odisha, India.

Objective: To compare the efficacy of intravenous acetaminophen and intravenous diclofenac sodium in the management of skeletal vaso-occlusive crisis among children with sickle cell disease.

Design: Single blind randomized controlled trial.

Setting: Tertiary care hospital. Read More

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In vivo muscle function and energetics in women with sickle cell anemia or trait: a P-magnetic resonance spectroscopy study.

J Appl Physiol (1985) 2021 03 10;130(3):737-745. Epub 2020 Dec 10.

Aix-Marseille Université, CNRS, CRMBM, Marseille, France.

Sickle cell anemia (SCA) is a genetic hemoglobinopathy associated with an impaired oxygen delivery to skeletal muscle that could alter ATP production processes and increase intramuscular acidosis. These alterations have been already reported in the Townes mouse model of SCA but the corresponding changes in humans have not been documented. In the present study, we used 31-phosphorus magnetic resonance spectroscopy to investigate in vivo the metabolic changes induced by a moderate-intensity exercise in twelve SCA patients, eight sickle cell trait (SCT) carriers, and twelve controls women. Read More

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The effect of dietary nitrate supplementation on the speed-duration relationship in mice with sickle cell disease.

J Appl Physiol (1985) 2020 09 23;129(3):474-482. Epub 2020 Jul 23.

Cardiovascular and Pulmonary Research Laboratory, Department of Medicine, University of Colorado Denver, Anschutz Medical Campus, Aurora, Colorado.

Sickle cell disease (SCD) causes exercise intolerance likely due to impaired skeletal muscle function and low nitric oxide (NO) bioavailability. Dietary nitrate improves hemodynamic and metabolic control during exercise in humans and animals. The purpose of this investigation was to assess the impact of nitrate supplementation on exercise capacity as measured by the running speed to exercise duration relationship [critical speed (CS)]in mice with SCD. Read More

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September 2020

Muscle structural, energetic and functional benefits of endurance exercise training in sickle cell disease.

Am J Hematol 2020 11 1;95(11):1257-1268. Epub 2020 Aug 1.

Laboratoire Interuniversitaire de Biologie de la Motricité, Université Savoie Mont Blanc, Chambéry, France.

Sickle cell disease (SCD) patients display skeletal muscle hypotrophy, altered oxidative capacity, exercise intolerance and poor quality of life. We previously demonstrated that moderate-intensity endurance training is beneficial for improving muscle function and quality of life of patients. The present study evaluated the effects of this moderate-intensity endurance training program on skeletal muscle structural and metabolic properties. Read More

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November 2020

Development of Algorithm for Clinical Management of Sickle Cell Bone Disease: Evidence for a Role of Vertebral Fractures in Patient Follow-up.

J Clin Med 2020 May 25;9(5). Epub 2020 May 25.

Section of Internal Medicine, Department of Medicine, University of Verona, 37134 Verona, Italy.

Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Read More

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Evaluation of the effectiveness of prophylactic oral vitamin D (cholecalciferol) in children with sickle cell disease.

Bone 2020 04 20;133:115228. Epub 2020 Jan 20.

Pediatric Hematology Unit, "Hospital General Universitario Gregorio Marañón", Facultad de Medicina, Universidad Complutense de Madrid, Spain; Instituto Investigación Sanitaria Gregorio Marañón, Madrid, Spain; Instituto Nacional de Investigación Biomédica en Enfermedades Raras (CIBERER), Instituto de Salud Carlos III, Madrid, Spain. Electronic address:

Background: Vitamin D (25(OH)D) deficiency has become an emerging public health problem due to its influence on skeletal and extraskeletal diseases. Bone health in patients with sickle cell disease (SCD) is especially compromised and they are more likely to have 25(OH)D deficiency than the general population. Despite this, there is little information on the efficacy of vitamin D3 (vitD3) prophylaxis and its role in improving bone mineral density (BMD) in this population. Read More

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Translating exercise benefits in sickle cell disease.

Blood 2019 12;134(25):2227-2229

Ann & Robert H. Lurie Children's Hospital of Chicago.

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December 2019

HbS/β+ thalassemia: Really a mild disease? A National survey from the AIEOP Sickle Cell Disease Study Group with genotype-phenotype correlation.

Eur J Haematol 2020 Mar 12;104(3):214-222. Epub 2019 Dec 12.

Unit of Pediatric Hematology and Oncology, Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.

Objectives: HbS/β+ patients' presence in Italy increased due to immigration; these patients are clinically heterogeneous, and specific guidelines are lacking. Our aim is to describe a cohort of HbS/β+ patients, with genotype-phenotype correlation, in order to offer guidance for clinical management of such patients.

Methods: Retrospective cohort study of HbS/β+ patients among 15 AIEOP Centres. Read More

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Beneficial effects of endurance exercise training on skeletal muscle microvasculature in sickle cell disease patients.

Blood 2019 12;134(25):2233-2241

Laboratoire Interuniversitaire de Biologie de la Motricité, Université de Lyon, Université Jean Monnet, Saint-Etienne, France.

Sickle cell disease (SCD) is a genetic hemoglobinopathy leading to 2 major clinical manifestations: severe chronic hemolytic anemia and iterative vaso-occlusive crises. SCD is also accompanied by profound muscle microvascular remodeling. The beneficial effects of endurance training on microvasculature are widely known. Read More

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December 2019

Urinary cross-linked carboxyterminal telopeptide, a bone resorption marker, decreases after vaso-occlusive crises in adults with sickle cell disease.

Blood Cells Mol Dis 2020 02 24;80:102369. Epub 2019 Oct 24.

Department of Medicine, Division of Hematology, University of Washington School of Medicine, Seattle, WA, United States of America; Washington Center for Bleeding Disorders at Bloodworks Northwest, University of Washington, Seattle, WA, United States of America.

People with sickle cell disease often report severe bone pain with repeated bouts of vaso-occlusive crises, but the extent of skeletal injury incurred during these painful episodes remain unclear. We sought to quantify bone degradation by comparing urinary concentrations of carboxyterminal cross-linked telopeptide of type I collagen (CTX-1), a well-described marker of bone resorption, in a prospective cohort of 52 adults with sickle cell disease enrolled in the Sickle Cell Pain Markers Study. We also questioned if changes in urinary CTX-1 concentrations correlated with changes in hemolysis and inflammatory markers measured both during and after resolution of a painful vaso-occlusive episode. Read More

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February 2020

Craniofacial and occlusal features of children with sickle cell disease compared to normal standards: a clinical and radiographic study of 50 paediatric patients.

Eur Arch Paediatr Dent 2020 Jun 17;21(3):303-311. Epub 2019 Oct 17.

Mahavir Surgical Hospital, Chandrapur, Maharashtra, India.

Purpose: To evaluate craniofacial and occlusal features of children with sickle cell disease and compare them with the normal healthy children.

Methods: A total of 50 children diagnosed with sickle cell disease (Group I) and 50 normal healthy children (Group II) between age 10 and 18 years were included in the study. Dental casts were obtained, occlusal traits were recorded and DAI and DHC-IOTN were calculated. Read More

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Effects of Individualized Treadmill Endurance Training on Oxidative Stress in Skeletal Muscles of Transgenic Sickle Mice.

Oxid Med Cell Longev 2019 24;2019:3765643. Epub 2019 Jul 24.

Interuniversity Laboratory of Human Movement Biology EA7424, Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon 1, Villeurbanne, France.

Oxidative stress is a key feature in the pathophysiology of sickle cell disease. Endurance training has been shown to reduce oxidative stress in the heart and the liver of sickle mice. However, the effects of endurance training on skeletal muscles, which are major producers of reactive oxygen species during exercise, are currently unknown. Read More

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January 2020

Contrast-enhanced ultrasound detects changes in microvascular blood flow in adults with sickle cell disease.

PLoS One 2019 5;14(7):e0218783. Epub 2019 Jul 5.

Department of Medicine, Medical College of Wisconsin, Milwaukee, WI, United States of America.

In patients with sickle cell disease (SCD), poor outcome measures compromise the potential success of clinical trials. Contrast-enhanced ultrasound (CEUS) is a technique that can non-invasively quantify deep tissue microvascular blood flow. We tested the hypothesis that CEUS of forearm skeletal muscle could be used to: 1) assess microvascular abnormalities that occur during vaso-occlusive crisis; and 2) test new therapies for SCD that are targeted to improving the status of the microcirculation. Read More

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February 2020

Weak and Winded.

N Engl J Med 2019 Jul;381(1):76-82

From the Departments of Neurology (I.L., K.N.) and Medicine (J.B.W., L.L.W.), University of Alabama at Birmingham, Birmingham.

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Sickle cell diseases: What can nuclear medicine offer?

Hell J Nucl Med 2019 Jan-Apr;22(1):2-3. Epub 2019 Mar 7.

Nuclear Medicine Unit, Interdisciplinary Department of Medicine, University of Bari "Aldo Moro", Bari, Italy.

Sickle cell disease (SCD) is the best known haemoglobinopathy, caused by a mutation substituting valina for glutamic acid at position 6 of the beta-globin chain of adult hemoglobin A, resulting in hemoglobin S (HbS). The homozygous HbS disease (HbSS), an autosomal recessive disorder, is the most common form and the Mediterranean area, along with sub-Saharian African and India, have the highest prevalence (1%-15%). In particular, Sicily with a prevalence of 2%-5%, is among the most interested regions. Read More

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Ischaemia-induced muscle metabolic abnormalities are poorly alleviated by endurance training in a mouse model of sickle cell disease.

Exp Physiol 2019 03 15;104(3):398-406. Epub 2019 Jan 15.

Aix-Marseille Univ, CNRS, CRMBM, Marseille, France.

New Findings: What is the central question of this study? The aim of this study was to evaluate the potential beneficial effects of endurance training during an ischaemia-reperfusion protocol in a mouse model of sickle cell disease (SCD). What is the main finding and its importance? Endurance training did not reverse the metabolic defects induced by a simulated vaso-occlusive crisis in SCD mice, with regard to intramuscular acidosis, mitochondrial dysfunction or anatomical properties. Our results suggest that endurance training would reduce the number of vaso-occlusive crises rather than the complications related to vaso-occlusive crises. Read More

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Exacerbated metabolic changes in skeletal muscle of sickle cell mice submitted to an acute ischemia-reperfusion paradigm.

Clin Sci (Lond) 2018 10 2;132(19):2103-2115. Epub 2018 Oct 2.

Aix-Marseille Univ, CNRS, CRMBM, Marseille, France.

Sickle cell disease (SCD) is characterized by painful vaso-occlusive crisis. While there are several metabolic abnormalities potentially associated with muscular ischemia-reperfusion cycles that could be harmful in the context of SCD, the metabolic consequences of such events are still unknown. Ten controls (HbAA), thirteen heterozygous (HbAS), and ten homozygous (HbSS) SCD mice were submitted to a standardized protocol of rest-ischemia-reperfusion of the left leg during which adenosine triphosphate, phosphocreatine, and inorganic phosphate concentrations as well as intramuscular pH were measured using phosphorous magnetic resonance spectroscopy (MRS). Read More

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October 2018

A microfluidic approach to study the effect of mechanical stress on erythrocytes in sickle cell disease.

Lab Chip 2018 09;18(19):2975-2984

Biologie Intégrée du Globule Rouge UMR_S1134, Inserm, Univ. Paris Diderot, Sorbonne Paris Cité, Univ. de la Réunion, Univ. des Antilles, INTS, 6 rue Alexandre Cabanel, 75015 Paris, France.

The human red blood cell is a biconcave disc of 6-8 × 2 μm that is highly elastic. This capacity to deform enables it to stretch while circulating through narrow capillaries to ensure its main function of gas exchange. Red cell shape and deformability are altered in membrane disorders because of defects in skeletal or membrane proteins affecting protein-protein interactions. Read More

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September 2018

The role of nitrite in muscle function, susceptibility to contraction injury, and fatigability in sickle cell mice.

Nitric Oxide 2018 11 14;80:70-81. Epub 2018 Aug 14.

Department of Perioperative Medicine, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD, 20892, USA. Electronic address:

Sickle cell disease (SCD) patients can have limited exercise capacity and muscle dysfunction characterized by decreased force, atrophy, microvascular abnormalities, fiber distribution changes, and skeletal muscle energetics abnormalities. Growing evidence suggests that in SCD there is alteration in nitric oxide (NO) availability/signaling and that nitrate/nitrite can serve as a NO reservoir and enhance muscle performance. Here, we examined effects of nitrite on muscle strength, exercise capacity, and on contractile properties of fast-(extensor digitorum longus, EDL) and slow-twitch (soleus) muscles in SCD mice. Read More

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November 2018

How Sickle Cell Disease Impairs Skeletal Muscle Function: Implications in Daily Life.

Med Sci Sports Exerc 2019 01;51(1):4-11

Inter-university Laboratory of Human Movement Sciences, University of Savoie Mont Blanc, Chambéry, FRANCE.

Sickle cell disease (SCD) is the most frequent life-threatening genetic hemoglobinopathy in the world and occurs due to the synthesis of abnormal hemoglobin S (HbS). hemoglobin S-containing red blood cells (RBC) are fragile, leading to hemolysis and anemia, and adhere to the endothelium, leading to hemorheological and hemodynamical disturbances. In its deoxygenated form, HbS may polymerize, leading to sickling of red blood cells and potentially to vasoocclusive crises. Read More

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January 2019

Muscle dysfunction is associated with poorer health-related quality of life in adults with sickle cell anaemia.

J Back Musculoskelet Rehabil 2019 ;32(1):43-53

Rehabilitation Sciences Post-Graduation Programme, Augusto Motta University, Rio de Janeiro, Brazil.

Background: It is known that episodes of microvascular obstruction and oxidative stress in sickle cell anaemia (SCA) can damage muscle tissue. As a consequence, deterioration in muscle function may potentially contribute to poor health-related quality of life (HRQoL) in subjects with SCA, particularly those who do not use long-term treatment.

Objectives: To evaluate muscle function in adults with SCA, to study the correlations between muscle function and HRQoL and to analyse the impact of hydroxyurea treatment. Read More

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